Idiopathic Intracranial Hypertension In Children Research Articles

The diagnostic value of MRI findings in pediatric idiopathic intracranial hypertension: a case-control study.

Idiopathic intracranial hypertension (IIH) is a rare medical condition in children. Based on the different radiological findings reported in various studies in pediatric IIH, this study was conducted to determine the diagnostic value of MRI findings in diagnosing IIH in children. In this retrospective study, the medical records of all children aged 1 to 18 years who visited Ghaem Hospital in Mashhad, Iran, between 2012 and 2022 and were diagnosed with IIH were gathered. Forty-nine cases of children with IIH and 48 control cases of children with the first unprovoked seizure with no indications of increased intracranial pressure for comparison were selected. Patient demographic information and MRI findings were extracted. The comparison between different MRI findings in the case and control groups was conducted using statistical tests. In the case group, the mean diameter of the subarachnoid space expansion around the optic nerve was 5.96 ± 1.21, compared to 4.79 ± 0.33 in the control group, with statistically significant difference (P < 0.001). All the patients with flattening of the posterior globe or transverse sinus stenosis were in the case group, and the frequency of these findings in the case group was significantly higher than in the control group (P < 0.001). The majority of patients (95.5%) classified under category 3 and 4 of empty sella were part of the case group, and the statistical test results indicated a significant difference between the two groups (P < 0.001). The optic nerve sheath diameter cut-off of 5.35 mm, when used for expansion of the subarachnoid space around the optic nerve, with a sensitivity of 82% and a specificity of 100% in diagnosing IIH. The most reliable diagnostic indicators for diagnosing IIH in children are perioptic subarachnoid space expansion with high sensitivity, and posterior globe flattening and transverse sinus stenosis with high specificity.

Consensus recommendations for the assessment and management of idiopathic intracranial hypertension in children and young people

BackgroundIdiopathic intracranial hypertension (IIH) is a potentially disabling condition. There is a lack of evidence and national guidance on how to diagnose and treat paediatric IIH, leading to variation in...

Pediatric Idiopathic Intracranial Hypertension: Clinical Presentations, Risk Factors, and Prognostic Indicator

Background: Idiopathic intracranial hypertension (IIH) is characterized by elevated cerebrospinal fluid pressure without space-occupying lesions, infections, or alterations in brain parenchyma. Diagnosing IIH in children poses a significant challenge for pediatricians, given the often nonspecific nature of clinical signs and symptoms. Objectives: Our study aims to evaluate the clinical presentations and potential risk factors among pediatric individuals diagnosed with IIH, considering the limited research in this particular domain. Methods: Clinical data from pediatric patients diagnosed with IIH who sought care at Tehran's primary referral children's hospitals were collected from 2013 to 2021, spanning eight years. These patients were subsequently contacted to follow up on the presence of persistent headaches and visual problems. Detailed records of their initial signs and symptoms were documented. To identify prognostic factors associated with persistent headaches and visual problems in pediatric IIH patients, binary logistic regression analysis was conducted. Results: A total of 81 pediatric patients were included in the study, with a mean age of 13.56 ± 4.404 years at the time of their IIH diagnosis. The most frequently reported clinical symptom among these patients was headache, observed in 85.2% of cases, followed by diplopia (50.6%), visual impairment (46.9%), and nausea with/without vomiting (44.4%). Furthermore, a substantial proportion of the patients were underweight (weight percentiles &lt; 3). Our analysis showed that male patients and those without strabismus experienced significantly more recurrent episodes of IIH (P = 0.013 and P = 0.013, respectively). Notably, recurrent episodes and higher weight percentiles emerged as predictive factors for future persistent visual problems within our study population (P = 0.032 and P = 0.045, respectively). Conclusions: Recurrence of IIH was significantly less in female patients and those with strabismus. Additionally, we found that both lower and higher weight percentiles, as well as the occurrence of recurrent episodes, served as predictive factors for the development of persistent visual problems. However, our model could not predict persistent headaches.

Predictors of disease course and long-term outcomes of idiopathic intracranial hypertension in children and adolescents.

This study provides insights into predictive factors for an unfavorable disease course, persistent headaches, and poor visual outcomes in patients with childhood IIH. Patients with persistent headaches may have a variant of a chronic pain syndrome warranting a different therapeutic approach. • Childhood-onset Idiopathic Intracranial hypertension (IIH) is a heterogenous disease. The knowledge on disease trajectory and long-term outcomes and its predictors is limited. • A higher opening pressure and factors suggestive of the metabolic syndrome predict an unfavorable disease coursewhereasfemale sex and disease recurrence are significantly associated with poor visual outcomes • A third of the patients diagnosed with IIH experience ongoing headachesdespite achieving favorable visual outcomes.This subset, characterized by lower disease-severity indicators at onset may represent a distinct subgroup warranting a different therapeutic approach.

Patient Delay, Lead Times, and Adherence to Diagnostic Guidelines in Children and Adolescents With Idiopathic Intracranial Hypertension

AimsIn a cohort of 45 children and adolescents diagnosed with idiopathic intracranial hypertension (IIH), our main aims were to investigate patient delay, lead time to final diagnosis, and adherence to current diagnostic guidelines. MethodsThis population-based, retrospective, single-center cohort study was performed at Uppsala University Childrenʼs Hospital, Sweden, a tertiary referral center for children and adolescents with rare and/or complicated neurologic disease. Patient data were retrieved from the local registries for patients filling the following criteria: age (0−17.99 yr), study period (2000−2020), and International Classification of Diseases code G93.2 (IIH). Medical records from pediatric, neuropediatric, ophthalmology, and neurosurgery departments were scrutinized. All included patients met the Friedman criteria. ResultsFifty-one percent of the patients sought medical advice within 1 month of symptom debut, 23% were seen within 1 to three months, and 26% after three months. A final diagnosis of IIH was reached within 48 hours in 60%, within two weeks in 80%, and within four weeks in 89% of patients. Visual fields, color vision, and complete ancillary laboratory investigations to exclude secondary etiologies were performed in 62%, 47%, and 59% of patients, respectively. ConclusionThe clinical presentation of IIH in children and adolescents may range from acute fulminant symptoms, to a more insidious or even chronic presentation with long-term headache. Although a majority of patients received a correct and prompt diagnosis, lead time to final diagnosis and adherence to diagnostic care guidelines might be improved. A higher awareness and knowledge of the condition may achieve this.

Idiopathic intracranial hypertension in children with obesity.

Obesity is an under-recognised risk factor for raised intracranial pressure in the paediatric population. The pathophysiology remains unclear. The aim of our study was to investigate the association between idiopathic intracranial hypertension (IIH) and weight in children. Patients diagnosed with IIH at a tertiary children's hospital were retrospectively identified between April 2017 and April 2019. Information regarding the patients' body mass index, presentation, investigation and treatment was collected and analysed. In total, 18 patients (M:F 7:11) were identified with a mean age of 11years (±3.3SD; range: 6-15years). The mean BMI was 30.3kg/m2 and mean BMI SDS was +2.5. Twelve (66.6%) patients presented with both headaches and eye signs. Three patients were asymptomatic, with papilloedema noted on routine optician review. Of the 18 patients, 15 were treated medically, two had long-term neurosurgical interventions and one patient was managed conservatively. The results show that the majority of children with obesity who develop IIH were female. Awareness regarding IIH secondary to obesity needs to be highlighted to ensure detailed clinical evaluation takes place so that raised intracranial pressure can be diagnosed and managed earlier, to avoid more serious complications such as permanent visual loss.

Pseudo tumor cerebri in children: Diagnosis and management approach

Idiopathic intracranial hypertension (IIH) is a rare neurological disorder in children. It is characterized by raised intracranial pressure in the presence of normal cerebrospinal fluid chemistry, normal neuroimaging, and no localizing signs on neurological exam (with the exception of cranial nerve VI palsy). We review clinical presentation, diagnosis and management of IIH in children.

Apparent diffusion coefficient echoplanar imaging maps of the optic nerves in childhood idiopathic intracranial hypertension.

Dueto motion artifacts, optic nerve (ON) findings of idiopathic intracranial hypertension (IIH) can easily be overlooked on T2-weighted (T2w) turbo spin-echo sequence. This study aimed to investigate the contribution of the apparent diffusion coefficient (ADC) map derived from the interleaved multi-shot (IMS) echoplanar imaging (EPI) to the ON findings of IIH in children. MRIs of 42 pediatric patients aged 3-17 years diagnosed with definite IIH according to modified Dandy criteria were retrospectively re-evaluated, between April 2018 and January 2021. Forty-two age- and sex-matched subjects with no IIH symptoms and reported as normal were included as a control group. ON sheath distance (ONSD) on the ADC map (p = .005) and vertical tortuosity (p = .030) were significant single MRI parameters for predicting IIH. Other single parameters were not statistically significant. Flattening of the posterior sclera (FPS) and ON protrusion (ONP) were observed on ADC maps more frequently than T2w (42.8% vs. 19% and 19% vs. 4.7%, respectively). From combined MRI parameters, the presence of at least one of ONP, FPS, or ONSD on ADC maps (p = .001) showed greater significance than the presence of T2w (p = .048). The predictive values of other MRI findings evaluated together were not statistically significant (p> .05). This study's results show that due to the short readout time and less sensitivity to motion, the ADC map obtained from IMS-EPI can contribute to orbital findings of IIH, in addition to T2w.

Visual outcomes in idiopathic intracranial hypertension in children

ObjectiveThe purpose of this study was to report the clinical characteristics and long-term visual outcomes in a cohort of children with idiopathic intracranial hypertension (IIH). DesignRetrospective, observational cohort study. ParticipantsConsecutive children who met the diagnostic criteria for definite IIH at a tertiary care pediatric hospital between 2009 and 2020. MethodsThe charts of pediatric patients with IIH were reviewed. The main outcome measure was long-term visual impairment, with an analysis of clinical features by age and risk factors for a poor visual outcome. ResultsThere were 110 children (75 females) with IIH. At presentation, younger children with IIH were less likely to present with headaches (p = 0.01) and more likely to be asymptomatic (p = 0.03). There was a strong association with female sex (p < 0.001) and higher body mass index (p < 0.001) in adolescents in comparison to younger children. Of the 90 patients with long-term visual outcome data, only 8 (9%) had evidence of mild visual impairment (1 loss of visual acuity, 7 loss of visual field) with no cases of severe visual impairment. On risk factor analysis, the only variable associated with a poor visual outcome was greater severity of papilledema at diagnosis. ConclusionsIn this large series of pediatric IIH, the long-term visual outcomes were favourable, with evidence of mild visual impairment in less than 10% of patients.

Paediatric Idiopathic Intracranial Hypertension (IIH)-A Review.

Paediatric idiopathic intracranial hypertension (IIH), is a rare but important differential diagnosis in children presenting with papilloedema. It is characterised by raised intracranial pressure in the absence of an identifiable secondary structural or systemic cause and is, therefore, a diagnosis of exclusion. In the adult population, there is a strong predilection for the disease to occur in female patients who are obese. This association is also seen in paediatric patients with IIH but primarily in the post-pubertal cohort. In younger pre-pubertal children, this is not the case, possibly reflecting a different underlying disease aetiology and pathogenesis. Untreated IIH in children can cause significant morbidity from sight loss, chronic headaches, and the psychological effects of ongoing regular hospital monitoring, interventions, and medication. The ultimate goal in the management of paediatric IIH is to protect the optic nerve from papilloedema-induced optic neuropathy and thus preserve vision, whilst reducing the morbidity from other symptoms of IIH, in particular chronic headaches. In this review, we will outline the typical work-up and diagnostic process for paediatric patients with suspected IIH and how we manage these patients.

Idiopathic intracranial hypertension in child

We present the case of a 12-year-old boy with bilateral papilledema, relating moderate symptomatology and without an important medical history. Ophthalmological examination revealed a significant alteration of visual acuity, an important papilledema and macular edema in both eyes. Further investigations excluded infectious and autoimmune diseases, intracranial masses and congenital affliction. Because of an elevated opening pressure in lumbar puncture procedure, the diagnosis of intracranial hypertension was confirmed. After two weeks of treatment with corticosteroids, carbonic anhydrase inhibitor and hyperosmotic drug, the patient had an important structural and functional ophthalmological improvement.

Management of idiopathic intracranial hypertension in children utilizing venous sinus stenting.

Venous sinus stenting (VSS) is an accepted and minimally invasive treatment for adult idiopathic intracranial hypertension (IIH) associated with lateral sinus stenosis (LSS). The efficacy and safety of venous sinus stenting (VSS) in children with IIH has not been established. This is a retrospective analysis of IIH patients 18 years of age or younger with LSS treated with VSS at our institution. Included patients have fulminant disease course or are refractory or intolerant to medical management. Eight patients were identified; 4 males and 4 females. Mean age is 13.4 years (range 4-18). All patients had severe headaches, 5 had blurred vision, 3 had diplopia and 3 had pulsatile tinnitus. Papilledema was present in 4 patients. Three patients had prior surgical procedures. Four patients were intolerant to medical management, 3 were refractory and 1 had fulminant course. Cerebral venography demonstrated severe stenosis of the dominant sinus in 6 patients and of bilateral co-dominant sinuses in 2 patients. Six patients had intrinsic stenosis and 2 had extrinsic stenosis. Venous sinus stenting (VSS) resulted in improvement of symptoms, papilledema and normalization of CSF opening pressure in 7 patients. No immediate complications were observed. Mean follow-up period is 21 months (range 6-42). Two patients required re-stenting; one responded well and the other had persistent symptoms and underwent subsequent surgical procedures of CSF diversion, suboccipital decompression and duraplasty which were also ineffective. VSS may provide a viable option for pediatric IIH patients who are intolerant to medication, have failed conservative management or prior surgical interventions, or present with fulminant disease.

Idiopathic Intracranial Hypertension in children: Are we missing something? (4546)

Idiopathic Intracranial Hypertension in children: Are we missing something? (4546)

Asymptomatic Versus Symptomatic Idiopathic Intracranial Hypertension in Children.

Idiopathic intracranial hypertension is a rare neurologic condition characterized by elevated intracranial pressure with normal cerebrospinal fluid analysis and neuroimaging. A subset of pediatric idiopathic intracranial hypertension patients are coincidentally found to have papilledema and elevated intracranial pressure without symptoms (eg, headache, visual blurring, tinnitus). This study aims to investigate the features of asymptomatic pediatric idiopathic intracranial hypertension. Retrospective case-control study of patients aged 0 to 18 years who received idiopathic intracranial hypertension diagnosis from 2005 to 2016. Subjects were included if they met established diagnostic criteria for idiopathic intracranial hypertension diagnosis. Subjects were classified as symptomatic if they presented with 1 symptom related to elevated intracranial pressure, and asymptomatic if no symptoms were present. Statistical analysis was performed to compare the 2 groups. 12 (22.6%) of 53 pediatric idiopathic intracranial hypertension subjects were asymptomatic. Compared to symptomatic idiopathic intracranial hypertension, asymptomatic idiopathic intracranial hypertension had younger age of onset, lower initial opening pressure on lumbar puncture, lower optic nerve edema grades bilaterally, lower likelihood of globe flattening on magnetic resonance imaging (MRI), and smaller required dose of acetazolamide for resolution of papilledema (all P < .05). Asymptomatic idiopathic intracranial hypertension is common among pediatric patients with papilledema and is an important disease entity that requires special clinical management. It may exist as a milder version of idiopathic intracranial hypertension that occurs in younger children, or as a precursor state that later evolves into symptomatic disease.

Clinical features of pediatric idiopathic intracranial hypertension.

Introduction: Adult idiopathic intracranial hypertension (IIH) is often linked to obesity, however, causes of IIH in children are not well understood. This project identifies potential risk factors and features of pediatric IIH.Methods: This study consisted of a retrospective chart review of patients ages 5–17 years who were seen at a tertiary care children’s hospital and diagnosed with IIH. Diagnostic criteria included the presence of papilledema, normal neurological exam, normal neuroimaging, normal cerebrospinal fluid composition, and an opening pressure of a lumbar puncture >28 mmHg.Results: Of the 26 cases of IIH, 19 met all diagnostic criteria for this study, while seven patients were probable IIH, as they lacked papilledema. Intracranial pressure ranged from 28 to 66 mmHg, with a mean of 40.23 mmHg (±10.74). Overall, 50.0% (95% CI: 29.9–70.1%) of IIH patients were obese, with patients 12 years of age and younger exhibiting an overall obesity rate of 30.7% and patients 13 years of age and older having an obesity rate of 69.2%. The overall allergy rate in this IIH patient population was 46.2% (95% CI: 26.6–66.6%).Conclusion: Obesity appears to have no association with IIH in younger cases, but it is a more common feature in older children. An autoimmune component may play a role in pediatric IIH, given the high rate of atopy observed in this pediatric IIH patient cohort. Because a diagnosis of IIH can have an absence of optic nerve edema, taking a detailed history and performing a thorough examination are keys to diagnosing IIH in the pediatric population.

Cranial morcellation decompression for refractory idiopathic intracranial hypertension in children.

Primary idiopathic intracranial hypertension (PIIH) in children is rare and has a poorly understood pathophysiology. It is characterized by raised intracranial pressure (ICP) in the absence of an identified brain lesion. Diagnosis is usually confirmed by the measurement of a high cerebrospinal fluid (CSF) opening pressure and exclusion of secondary causes of intracranial hypertension. Refractory PIIH may lead to severe visual impairment. The purpose of this study was to evaluate a cranial morcellation decompression (CMD) technique as a new surgical alternative to stabilize intracranial pressure in PIIH. A literature review was carried out, disclosing only 7 pediatric cases of PIIH treated with surgical skull expansion. In addition, we describe here one case of our own experience treated by CMD. CMD surgery is a safe and effective option to control refractory PIIH in selected patients.

Idiopathic Intracranial Hypertension in Children and Adolescents: An Update.

Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri syndrome (PTC) is a serious neurological disorder that can lead to irreversible visual loss. Predominantly a disorder affecting women in reproductive years, the pediatric population is not spared. In the past few years, the condition has been redefined, due to new accepted values for opening pressure in children and advances in neuroimaging. Emerging techniques in ophthalmology are being increasingly used to monitor disease in these patients. And, although the treatment tools have not changed in several years, important evidence for efficacy for acetazolamide finally came to light in recent years in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). This review article provides an overview on recent advances in diagnosis, evaluation and treatment of IIH.

Outcomes of ventriculoperitoneal shunt insertion in the management of idiopathic intracranial hypertension in children

PurposeThe ventriculoperitoneal (VP) shunt has become the procedure of choice for treatment of idiopathic intracranial hypertension (IIH). We aimed to assess the efficacy of frameless stereotactic placement of VP shunts for the management of medically resistant IIH in children and to assess the role of gender and obesity in the aetiology of the condition.MethodsThis is a retrospective analysis of the case notes of 10 patients treated surgically at the University Hospital of Wales in Cardiff, from May 2006 to September 2012.ResultsVP shunts were successful in relieving headache, papilloedema and stabilising vision. No sex predilection was identified, and increased BMI was a feature throughout the population, regardless of age.ConclusionsNeuronavigated VP shunt insertion is an effective mode of treatment for medically resistant IIH in children. The aetiological picture in children does not seem to be dominated by obesity, as in adults. Literature on childhood IIH is sparse, and larger scale, comparative studies would be of benefit to treating clinicians.

Imaging Features of Idiopathic Intracranial Hypertension in Children.

Magnetic resonance imaging (MRI) signs of elevated intracranial pressure and idiopathic intracranial hypertension have been well characterized in adults but not in children. The MRIs of 50 children with idiopathic intracranial hypertension and 46 adults with idiopathic intracranial hypertension were reviewed for optic nerve head protrusion, optic nerve head enhancement, posterior scleral flattening, increased perioptic cerebrospinal fluid, optic nerve tortuosity, empty or partially empty sella, tonsillar herniation, enlargement of Meckel's cave meningoceles, and transverse venous sinus stenosis(TSS). Compared to adolescents (11-17 years, n = 40) and adults (>17 years, n = 46), prepubescent children (<11 years, n = 10) had lower frequencies of scleral flattening (50% vs 89% and 85%, P = .02), increased perioptic cerebrospinal fluid (60% vs 84% and 89%, P = .08), optic nerve tortuosity (20% vs 46% and 59%, P = .07), empty or partially empty sella (56% vs 78% and 93%, P = .007), and TSS (67% vs 93% and 96%, P = .04). Children with idiopathic intracranial hypertension have similar MRI findings as adults, but they are less frequent in prepubescent children.

Visual Outcome and Recurrence Rate in Children With Idiopathic Intracranial Hypertension.

The purpose of this retrospective study was to evaluate the visual outcome and recurrence rate of idiopathic intracranial hypertension in children. The study included 68 patients who were diagnosed with idiopathic intracranial hypertension according to the modified Dandy criteria. Permanent visual impairment was rare. Three percent remained with mild visual impairment, 4% with minimal visual field defects, and only 1 patient had severe visual impairment. However, 26% had either a prolonged course of disease or a recurring condition. Higher cerebrospinal fluid opening pressure was the only clinical predictor at presentation (P = .04). Recurrence rate was 18%, and in most cases, the second episode occurred during the first year after remission. There was no significant difference between the group of patients with only 1 episode and the group of patients with more than 1 episode. We suggest long-term follow-up after remission, for at least a year, for all children with idiopathic intracranial hypertension.